Isolated noncompaction of the ventricular myocardium: contemporary diagnosis and management

Abstract

Noncompaction of the ventricular myocardium is a rare form of cardiomyopathy that has been described since the early 1990s. However, noncompaction remains frequently overlooked, in part due to the limited awareness of its unique clinical and imaging characteristics. Contemporary diagnosis has been facilitated by the introduction of specific morphologic criteria by echocardiography and cardiac magnetic resonance. Management issues revolve around the management of heart failure, arrhythmias, and thromboembolic events in order to prevent the significant morbidity and even mortality that has been associated with this entity. Finally, the genetics of noncompaction have been diverse and an issue of clinical importance as it relates to screening of first-degree relatives of affected patients. Two recent cases are presented and many of the contemporary issues in diagnosis and management, based on an extensive review of the literature, are addressed.